Pulmonary manifestations in inflammatory bowel disease, both in Crohn disease and ulcerative colitis, are well documented. Best characterized and seen more often in Crohn disease than in ulcerative colitis, pulmonary manifestations can include bronchitis, bronchiectasis, bronchiolitis obliterans, organizing pneumonia, nodular suppuration, pulmonary vasculitis, and noninfectious granulomatous inflammation. Granulomatous inflammation in patients with ulcerative colitis has also been reported associated with azathioprine treatment. On biopsy, granulomas of various sizes can be seen in random distri-bution both within the interstitium as well as near or around bronchioles. The granulomas tend to be fairly well formed, surrounded by multinucleated giant cells and without central necrosis. The differential diagnosis of granulomatous inflammation can be extensive and includes infection, drugs, sarcoidosis, and hypersensitivity pneumonia. If there is an associated organizing pneumonia present or features of vasculitis, the diagnosis of pulmonary manifestation of inflammatory bowel disease can be difficult on a biopsy. Rarely in patients with inflammatory bowel disease, coexistence of sarcoidosis or a “true” vasculitis, such as Churg-Strauss syndrome, Wegener granulomatosis, and microscopic polyangiitis, have been reported. Good clinical history and correlation with the pathologic findings are key to the diagnosis. Microbiologic cultures, special stains for organisms, serum antineutrophil cytoplasmic antibody (ANCA), and angiotensin-converting enzyme (ACE) studies are recommended to aid in the differential diagnosis.